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Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS)
  1. Pavla Dolezalova1,
  2. Fiona E Price-Kuehne2,
  3. Seza Özen3,
  4. Susanne M Benseler4,
  5. David A Cabral5,
  6. Jordi Anton6,
  7. Jürgen Brunner7,
  8. Rolando Cimaz8,
  9. Katheleen M O'Neil9,
  10. Carol A Wallace10,
  11. Nicholas Wilkinson11,
  12. Despina Eleftheriou2,
  13. Erkan Demirkaya12,
  14. Marek Böhm1,
  15. Petra Krol1,
  16. Raashid A Luqmani13,
  17. Paul A Brogan2
  1. 1Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, Charles University in Prague, 1st Faculty of Medicine and General University Hospital in Prague, Czech Republic
  2. 2Institute of Child Health and Great Ormond Street Hospital For Children NHS Foundation Trust, UCL, London, UK
  3. 3Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
  4. 4Department of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Canada
  5. 5Department of Pediatric Rheumatology, BC Children's Hospital and University of British Columbia, Vancouver, Canada
  6. 6Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain
  7. 7Innsbruck Medical University, Innsbruck, Austria
  8. 8AOU Meyer, Firenze, Italy
  9. 9University of Oklahoma Health Sciences Center, Oklahoma City, USA
  10. 10Department of Pediatric Rheumatology, University of Washington and Seattle Children's Hospital, Seattle, USA
  11. 11Nuffield Orthopaedic Centre NHS Trust, Oxford, UK
  12. 12Gulhane Military Medical Faculty, School of Medicine, Ankara, Turkey
  13. 13Department of Rheumatology, NIHR Musculoskeletal Biomedical Research Unit, University of Oxford, Oxford, UK
  1. Correspondence to Dr Pavla Dolezalova, Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, Charles University in Prague, 1st Faculty of Medicine and General University Hospital in Prague, Ke Karlovu 2, 12800 Praha 2, Czech Republic; dolezalova.pavla{at}vfn.cz

Abstract

Background Rare chronic childhood vasculitides lack a reliable disease activity assessment tool. With emerging new treatment modalities such a tool has become increasingly essential for both clinical practice and therapeutic trials to reproducibly quantify change in disease state.

Objective To develop and validate a paediatric vasculitis activity assessment tool based on modification of the Birmingham Vasculitis Activity Score (BVASv.3).

Methods A paediatric vasculitis registry was reviewed to identify clinical features missing in the BVASv.3. A modified nominal group technique was used to develop a working version of the Paediatric Vasculitis Activity Score (PVAS). Prospective validation provided tool reliability, reproducibility and responsiveness to change. Training of assessors was done according to the BVAS principles.

Results BVAS items were redefined (n=22) and eight paediatric items added in Cutaneous (n=4), Cardiovascular (n=3) and Abdominal (n=1) sections. The final PVAS has 64 active items in nine categories. The principles of new/worse and persistently active disease were retained as were the overall score and weighting of categories. The median PVAS in 63 children with systemic vasculitis was 4/63 (0–38/63). There was a high interobserver agreement for the overall as well as for subsystem scores (linear-weighted-κ ≥0.87). PVAS correlated with physician's global assessment (p<0.01); treatment decision (p=<0.01) and erythrocyte sedimentation rate (ESR) (p=0.01). In response to treatment, 15/19 patients assessed demonstrated a significant fall in PVAS (p=0.002), with good agreement among assessors for this change.

Conclusions The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.

  • Systemic vasculitis
  • Disease Activity
  • Outcomes research
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